- Polke, Markus;
- Kondoh, Yasuhiro;
- Wijsenbeek, Marlies;
- Cottin, Vincent;
- Walsh, Simon;
- Collard, Harold;
- Chaudhuri, Nazia;
- Avdeev, Sergey;
- Behr, Jürgen;
- Calligaro, Gregory;
- Corte, Tamera;
- Flaherty, Kevin;
- Funke-Chambour, Manuela;
- Kolb, Martin;
- Krisam, Johannes;
- Maher, Toby;
- Molina Molina, Maria;
- Morais, Antonio;
- Moor, Catharina;
- Morisset, Julie;
- Pereira, Carlos;
- Quadrelli, Silvia;
- Selman, Moises;
- Tzouvelekis, Argyrios;
- Valenzuela, Claudia;
- Vancheri, Carlo;
- Vicens-Zygmunt, Vanesa;
- Wälscher, Julia;
- Wuyts, Wim;
- Bendstrup, Elisabeth;
- Kreuter, Michael
Background: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a severe complication associated with a high mortality. However, evidence and guidance on management is sparse. The aim of this international survey was to assess differences in prevention, diagnostic and treatment strategies for AE-IPF in specialised and non-specialised ILD centres worldwide. Material and Methods: Pulmonologists working in specialised and non-specialised ILD centres were invited to participate in a survey designed by an international expert panel. Responses were evaluated in respect to the physicians institutions. Results: Three hundred and two (65%) of the respondents worked in a specialised ILD centre, 134 (29%) in a non-specialised pulmonology centre. Similarities were frequent with regards to diagnostic methods including radiology and screening for infection, treatment with corticosteroids, use of high-flow oxygen and non-invasive ventilation in critical ill patients and palliative strategies. However, differences were significant in terms of the use of KL-6 and pathogen testing in urine, treatments with cyclosporine and recombinant thrombomodulin, extracorporeal membrane oxygenation in critical ill patients as well as antacid medication and anaesthesia measures as preventive methods. Conclusion: Despite the absence of recommendations, approaches to the prevention, diagnosis and treatment of AE-IPF are comparable in specialised and non-specialised ILD centres, yet certain differences in the managements of AE-IPF exist. Clinical trials and guidelines are needed to improve patient care and prognosis in AE-IPF.